Inheriting UHS: Uncombable Hair Syndrome

Definition of UHS

UHS, commonly known as Uncombable Hair Syndrome usually makes an appearance between 3 months to 12 years of age. It is also called “Pili trianguli et canaliculi” and “Spun glass hair.” The hair fibre is inflexible, refusing to lie flat against the scalp and the result is brittle, coarse, straw-like textured hair which cannot be combed flat. Since hair is dependent on Keratin and is similar in its basic structure to teeth and nails, problems such as brittle nails and tooth enamel are part of the parcel.

In UHS, the affected hair looks like spun glass strands growing haywire in berserk directions and is difficult to control. While the American Journal considers this a rare syndrome, many individuals have experienced the problem at early childhood, as teenagers and also as adults. While some have been lucky enough to outgrow the frizzy hair, many others are still dealing with the problem. In 1973, it was first described clinically and depicted in a German fairy tale of Struwwel Peter, a boy with messy hair and symptoms of Mental retardation.

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Genes and UHS

As defined by the American Journal of Medical Genetics, “Uncombable or spun-glass hair is an uncommon condition in which the hair is “unmanageable” and has a distinct appearance on scanning electron microscopy. The hair is usually grossly abnormal in infancy and childhood, but may become normal later in life. Although dominant inheritance has been observed, most cases have been sporadic. Both recessive and dominant transmission with incomplete penetration have been suggested as modes of inheritance. “

They also report the occurrence of this condition in a young girl, her brother, and her father. Although the sister and her brother had characteristically uncombable hair, their father appeared normal and denied any history of hair abnormality. However, the characteristic hair morphology was observed on scanning electron microscopy in all 3 relatives, documenting dominant transmission and complete penetration of the gene in this family.

Uncombable Hair Syndrome is within a group of over 100 Genetic abnormalities known as Ectodermal Dysplasia that affect the skin, hair, teeth and nails of individuals. The abnormality in the hair structure and the stiffness of the uncombable hair is due to the combination of Abnormal fibrous protein patterns and the triangular form of the hair shaft in cross section, resulting from premature keratinization of the inner root sheath.
Usually you can run your fingers through your hair and will easily bend if you comb it. In this case however, affected Hair is dry, curly and brittle. They are spangled or shimmery in appearance, often due to the reflection of light off the irregular surface of the hair shafts.

UHS Relief- BIOTIN

Biotin, the wonder drug, offers relieve to sufferers of the Uncombable Hair syndrome. Since it is one of the eight essential vitamins that comprise the Vitamin B-complex, Biotin promotes hair growth and is essential for maintaining healthy hair. Also known as Vitamin H, Biotin is a natural resource offering a break from tangling and uncombable hair and Nutritionists believe that people with certain Biotin deficiencies tend to have dry, stringy and dull hair. However the good news is that you can deliver biotin to your hair in various easier methods other than through ingestion and/or injections. Commonly used in Shampoos and Conditioners, Biotin penetrates and expands the hair shaft resulting in thickening of the hair cuticle and new healthier, richer, combable hair.